BACKGROUND: Soft tissue sarcomas (STS) are a group of anatomically and histologically diverse tumours. They account for about 1% of adult malignancies, and about 50% of patients diagnosed with sarcoma eventually die of the disease. These tumours are grouped together because of shared biological characteristics and treatment responses. METHODS: A review of the key literature on STS was undertaken, complemented by data taken from the prospectively accumulated database of 3442 patients treated for STS at Memorial Sloan-Kettering Cancer Center (MSKCC) from July 1982 to December 1997. RESULTS: Despite advances in knowledge of the molecular genetics of STS, the aetiology in most cases remains elusive. Management has been gradually refined over the last two decades. Core biopsy usually provides enough material for diagnosis. Computed tomography and magnetic resonance imaging provide a similar amount of information, which is usually adequate for surgical planning. Amputation as standard treatment for extremity lesions has been replaced by limb-sparing surgery in about 90% of patients. The role of adjuvant radiation has been further defined: it has been shown to be unnecessary for completely excised tumours less than 5 cm in diameter, but effective in decreasing local recurrence for others. Adjuvant chemotherapy has been controversial for many years. Recent meta-analysis of randomized trial results has found a small reduction in local and distant relapse, and a trend to better overall survival. Recurrent or metastatic disease should be completely excised if possible. CONCLUSIONS: Soft tissue sarcoma requires multidisciplinary care tailored to the individual patient and tumour for optimal results.
